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July 1994

Incontinentia Pigmenti Achromians-Like Depigmentation in the Mother of a Baby With Typical Incontinentia Pigmenti

Author Affiliations

Department of Dermatology Okayama University Medical School Okayama 700, Japan

Arch Dermatol. 1994;130(7):936-938. doi:10.1001/archderm.1994.01690070132026

We baby girl who presented with a typical incontinentia pigmenti (IP) and her mother who presented with bizarre-shaped, smooth-surfaced, depigmented lesions. The clinical relationship of these two patients is discussed.

Report of Cases.  A 2-month-old girl, born prematurely, presented with vesicles and pigmentation. At the age of 4 days, erythema, vesicles, and pustules appeared on her legs and trunk along Blaschko's lines. The lesions gradually spread, sparing her face. During the following 2 months, these lesions were followed by crusts, verrucous papules, and bizarre-shaped brown macules (Figure 1). Her 3-year-old sister did not have similar disease. Her mother had hypopigmented streaks and whorls. Her parents said that the mother had brownish macules on her legs in childhood. We further substantiated details obtained from her parents who said that the mother had never had vesicles or verrucous lesions in infancy. She had suffered from the Wolff-parkinson-White syndrome and had had hypertension

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